Transfusion medicine
Transfusion transmitted disease
Variant Creutzfeldt-Jakob
Author: Huy P. Pham, M.D., M.P.H.
Last author update: 1 September 2011
Last staff update: 26 December 2021
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Transfusion transmitted disease-Variant+Creutzfeldt-Jakob
Cite this page: Pham H. Variant Creutzfeldt-Jakob. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/transfusionmedvcj.html. Accessed April 19th, 2024.
Definition / general
- Prion disease results from the benign form of a prion protein changing to an insoluble protease resistant form → formation of plaques in brain
- Caused by prion protein that assumes an abnormal configuration, which promotes change to abnormal configuration of other proteins
Three forms of classic CJD:
- Sporadic (85%)
- Hereditary
- Acquired (through corneal transplants, pituitary gland derived growth hormone, dura mater transplants, inadequately sterilized brain electrodes)
- vCJD is human equivalent of "mad cow disease", due to epidemic of bovine spongiform encephalopathy, possibly due to prion in cattle entering human food supply
- In 1995, vCJD was documented in U.K.; transmission was through food chain
vCJD symptoms:
- Behavioral change, cerebellar ataxia and dementia; death in 7 - 38 months
- vCJD is rarely transmitted by transfusions of blood components but not plasma products
- As of 2007, in U.K., 66 individuals received transfusion products from donors who later developed vCJD; 3 have developed symptoms of vCJD, 1 had no symptoms of vCJD but evidence of abnormal prion proteins at autopsy for unrelated death
- In U.S., donors who have resided in U.K. or Europe are deferred
- Excluding transfusion recipients from donating blood is unlikely to change epidemiology of disease (Emerg Infect Dis 2007;13:89)
- References: epidemiologic study (Vox Sang 2006;91:221), Medscape: Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy [Accessed 27 October 2017], Centers for Disease Control and Prevention: Variant Creutzfeldt-Jakob Disease (vCJD) [Accessed 27 October 2017]
Case reports
Variant Creutzfeldt-Jakob
- United Kingdom - confirmed (Lancet 2006;368:2061)
- Possible transmission (Lancet 2004;363:417)
- Preclinical disease in patient with unrelated death (Lancet 2004;364:527)
